Practical insights into chronic management of hepatic Wilson’s disease

Table 2 Suggested clinical and biochemical chronic monitoring of patients with Wilson’s disease

Clinical and biochemical monitoring of Wilson’s disease
Clinical monitoring	Biochemical monitoring
Abdominal US (1/yr in non-cirrhotic, 1/6 mo in cirrhotic patients)	NCC = total serum copper concentration (in μg/dL; serum copper in μmol/dL × 63.5 = serum copper in μg/dL) - 3.15 × holo-ceruloplasmin in mg/dL
Neurological assessment (using the Unified Wilson’s Disease Rating Scale) at every follow-up visit	24-h urine copper excretion
Identification of possible side effects of anti-copper drugs	Liver enzymes and liver function tests (aspartate aminotransferase, alanine aminotransferase, γ-glutamyl-transferase, alkaline phosphatase, bilirubin, international normalized ratio, and albumin) creatinine, and complete blood count
Gastroscopy in cirrhotic patients, when appropriate	For patients treated with DPA and trientine: 24 h-urine protein test, anti-nuclear antibodies
Transient elastography (1/yr in non-cirrhotic patients)	For patients treated with zinc: 24-hour urine zinc excretion
Central bone density scan at diagnosis, then individualise follow-up
If non-compliance is suspected: Slit-lamp examination to search for Kayser-Fleischer rings, brain MRI
For patients treated with DPA: skin biopsy at 10 yr from treatment initiation

DPA: D-penicillamine; MRI: Magnetic resonance imaging; NCC: Non-ceruloplamin-bound copper; US: Ultrasound.